Due to genetic causes the mucociliary apparatus is defective in both patients with cystic fibrosis (CF) and in patients with primary ciliary dyskinesia (PCD). The defect results in acute and chronic infections in the respiratory tract. The mucociliary apparatus is the "purification apparatus" in the respiratory tract. It consists of mucus and tiny hairs (cilia) that "sweep" mucus with bacteria and viruses away, thereby preventing infection.
Chronic pulmonary infection with the bacteria P. aeruginosa (Pa) is the main reason for the increased morbidity and premature death in CF patients. The bacteriology in PCD patients is more diverse but a large proportion of the patients are also infected with Pa.
Commonly, CF patients are intermittently infected with Pa in childhood but over time the infection turns chronic and requires daily antibiotics for life.
CF patients often suffer from chronic sinusitis and Pa probably colonize the sinuses before the patients’ lungs are infected. The sinuses may function as a bacterial reservoir for recurrent lung infection and thereby play a crucial part in the establishment of the chronic, feared and dangerous lung infection.
We have demonstrated that minimal invasive sinus surgery (FESS), endoscopically through the nostrils, and intensive follow-up in several cases have been able to eradicate the bacteria from the sinuses in CF patients. The results are enhanced quality of life and decreased lung inflammation and lung destruction.
Patients with PCD accumulate secretions in the airways due to a defect in their cilia and suffer from chronic sinusitis. Currently, there exists no scientific evidence for the treatment of sinus problems in PCD patients; we suspect that the PCD patients are undertreated. Our aim is to establish an improved and evidence based treatment for these patients.
The Ph.d. project consists of three parts.
Systematically, we will examine the bacteriology in both the upper and lower airways in PCD patients. Around 100 patients are followed in the outpatient clinic at Rigshospitalet. This has not previously been studied in PCD patients. The information will help us organize further treatment: We wish to identify patients who are infected in the nose and sinuses with Pa or other lung pathogenic bacteria, so treatment can be initiated.
Our hypothesis is that sinus surgery with intensive follow up can eradicate pathogenic bacteria from the sinuses and enhance quality of life in patients with PCD. This is examined by adapting the methods used in CF patients and developing the interdisciplinary collaboration we have established working with CF patients for decades. We will operate approximately 20 PCD patients with symptoms of chronic sinus inflammation and evaluate the effect with standardized questionnaires and microbiologically by cultures.
In the nasal cavity and in saliva we have measured high levels antibodies against Pa early in the course of the infection. We want to finally prove that antibodies measured in nasal secretions or saliva can be used to easily and early diagnose Pa-sinusitis in CF and PCD patients. Hopefully, based on this easy and non-invasive test, we can initiate antibiotic treatment before the bacteria infects the lungs.